Pericardial effusion (PE) confers a poor prognosis in non-congenital pulmonary arterial hypertension (PAH). The clinical significance and prognostic implications of PE in Eisenmenger syndrome (ES) is much less well characterised. Accordingly, we aimed to define the frequency, echocardiographic characteristics, natural history and clinical sequelae of PE in those with ES.

We retrospectively studied 55 consecutive individuals presenting since 2000, age >18 years, with ES. Analysis of clinical notes, transthoracic echocardiograms and vital status was undertaken via local records and the National Death Index.

Mean age was 38 years (11.4SD), 22% had Down syndrome and the mean follow-up time was 14.7 years (13.4SD). Nine patients (16%) had a PE during their follow-up period with the majority being small (56%). No PE exhibited clinical or echocardiographic signs of tamponade and none required pericardiocentesis. At the time of last review, 42% of individuals had died. The presence of PE was not associated with mortality (p=0.83).

Pericardial effusion occurs relatively frequently in ES but is usually small and with no evidence of haemodynamic compromise. Pericardial effusion in this setting is not related to mortality.